Immune-mediated lung diseases: A narrative review
PAH Treatment Uptravi Leads To Improvements, Registry Data Show
Most adults with pulmonary arterial hypertension (PAH) who switched to Uptravi (selexipag) from other medications in its class achieved stabilization or a lessening of disease severity, according to an analysis of real-world treatment data from the SPHERE registry.
Clinical outcomes were also generally good for Uptravi-treated PAH patients with co-existing mental health conditions.
The analyses were presented a pair of poster presentations sponsored by developer Johnson & Johnson at the American College of Chest Physicians (CHEST) annual meeting, held in Boston Oct. 6-9.
Uptravi is an approved PAH therapy belonging to a class of medications called prostacyclin pathway agents (PPAs), which mimic the activity of prostacyclin, a naturally occurring molecule that helps blood vessels relax and widen. There are several other PPAs approved for PAH, which have various modes of administration.
SPHERE (NCT03278002) was designed to collect real-world data from patients who were being treated with Uptravi in the U.S. Clinical data were collected at routine clinical visits for up to 1.5 years. Previous registry analyses showed that most of the 759 adults with PAH who were enrolled in the registry experienced stabilizations or improvements in clinical status after 1.5 years.
Switching therapiesIn a poster titled, "Characteristics and outcomes of patients with pulmonary arterial hypertension transitioning to selexipag from another prostacyclin pathway agent in the SPHERE registry," researchers analyzed outcomes from registry participants who switched to Uptravi from another prostacyclin agent.
Of 759 patients enrolled in SPHERE, 124 (16%) had previously been on another PPA, including inhaled (47%), injectable (42%), or oral (11%) agents. They started on Uptravi a median of 5.8 years after their PAH diagnosis.
Of these 124 people, with a median age of 59, 84% were female and 71% were white. The majority (57%) had idiopathic PAH.
Most patients (79%) were in World Health Organization (WHO) functional class II or III at the time they entered the SPHERE registry. WHO functional class categorizes patients based on how severely PAH affects their physical function and exercise capacity, with classes II and III generally reflecting moderate limitations.
Most patients were in the lowest (55%) or intermediate (26%) risk category for REVEAL 2.0, which classifies patients' risk of death based on a number of clinical factors.
The overall survival rate after 1.5 years in this registry subgroup was 89%, with 85% of the 58 patients with evaluable data experiencing a stabilization or improvement in WHO functional class.
Reasons for permanently stopping Uptravi included treatment-related side effects (21 people), disease progression-related side effects (12 people), or other reasons (11 people). Eight people died.
Overall, "carefully selected patients may be able to successfully transition to [Uptravi] and reach stable or improved disease," the researchers wrote.
Mental healthIn another poster, "Characteristics and outcomes of patients with pulmonary arterial hypertension and self-reported mental health comorbidities in SPHERE (SelexiPag: tHe usErs dRug rEgistry)," scientists discussed outcomes from SPHERE registry participants who also self-reported having mental health conditions.
Of the 759 enrolled SPHERE participants, about a third — 32%, or 246 people — reported mental health issues, including depression (22.9%), anxiety or anxiety disorder (14.7%), or bipolar disorder (1.2%). All but one of these individuals reported receiving medical treatment for their mental health disorders.
Data showed that a lower proportion of patients with mental health conditions were receiving dual PAH therapy at the time of SPHERE enrollment compared with patients without such conditions (50% vs. 58%).
They were also less likely to have received a combination of an endothelin receptor antagonist therapy and a phosphodiesterase inhibitor therapy (34% vs. 45%), which are two other main PAH treatment classes.
Moreover, those with mental health conditions started Uptravi later in their disease course, a median of 3.3 years after their diagnosis vs. A median of 2.6 years post-diagnosis in those who didn't report mental health problems.
In terms of clinical outcomes, patients with mental health conditions generally did as well as those without them. Most patients in both groups (62%) had a stable WHO functional class as well as a stable REVEAL risk categorization (60% of those with mental health conditions and 62% of those without them).
The group with mental health problems had a median time to the first hospitalization of 11 months, compared with 16 months in the other group.
Survival rates after 1.5 years were 85.6% for patients with mental health conditions and 91.1% for those without them. After three years, survival rates were 77% and 84% for those with and without mental health conditions, respectively.
"This analysis should reassure healthcare providers that patients with mental health comorbidities can achieve favorable outcomes with [Uptravi]," the researchers wrote.
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AI Breakthrough In Pulmonary Hypertension Diagnosis: CHEST 2024
PULMONARY hypertension (PH) is a progressive and potentially fatal condition that can go undetected until it reaches advanced stages. Current diagnostic methods, such as transthoracic echocardiography (TTE), lack both sensitivity and specificity and may require invasive right heart catheterization to confirm a diagnosis. However, a groundbreaking study presented at the CHEST Annual Meeting 2024 in Boston, held from October 6 to 9, introduces a multimodal fusion model (MMF-PH) that could transform the detection of PH.
This innovative deep learning algorithm integrates data from electronic health records, including chest radiographs, ECG, demographics, and TTE findings, to deliver more accurate results than traditional TTE screening methods alone. The study found that MMF-PH demonstrated superior diagnostic performance across various patient groups, showing great promise for clinical use.
The research analyzed a dataset of 4,576 patients with unconfirmed but suspected PH at Beijing Fuwai Hospital, China, between 2019 and 2021. Of these, 2,451 cases were confirmed using right heart catheterization. The algorithm was rigorously trained and validated using both retrospective and prospective data, including external datasets from other medical institutions.
Dr. Zhihua Huang, from Chinese Academy of Medical Sciences and Fuwai Hospital said of the algorithm: "This multimodal deep learning algorithm enables early, accurate identification of pulmonary hypertension and its subtypes, potentially reducing the need for invasive procedures. It has the potential to significantly improve patient management and outcomes while reducing healthcare costs."
With its potential to improve early diagnosis and guide personalized treatments, the MMF-PH algorithm could revolutionize the clinical management of pulmonary hypertension, offering a less invasive, AI-driven solution.
Reference: Huang Z et al. Revolutionizing pulmonary hypertension detection: a multicenter study on a comprehensive multimodal deep learning approach. Abstract 4029. CHEST Annual Meeting. October 6-9, 2024.
Anaya MalikAMJ
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